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Mosby is an affiliate of Elsevier Inc.

© 2008, Elsevier Inc. All rights reserved.

First published 2008

No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission of the Publishers. Permissions may be sought directly from Elsevier’s Health Sciences Rights Department, 1600 John F. Kennedy Boulevard, Suite 1800, Philadelphia, PA 19103-2899, USA: phone (+1) 215 239 3804, fax (+1) 215 239 3805, or e-mail healthpermissions@elsevier.com. You may also complete your request online via the Elsevier homepage (http://www.elsevier.com) by selecting ‘Support and Contact’ and then ‘Copyright and Permission’.

ISBN 978-0-323-04456-1

British Library Cataloguing in Publication Data

A catalogue record for this book is available from the British Library

Library of Congress Cataloging in Publication Data

A catalog record for this book is available from the Library of Congress

Notice

Medical knowledge is constantly changing. Standard safety precautions must be followed, but as new research and clinical experience broaden our knowledge, changes in treatment and drug therapy may become necessary or appropriate. Readers are advised to check the most current product information provided by the manufacturer of each drug to be administered to verify the recommended dose, the method and duration of administration, and contraindications. It is the responsibility of the practitioner, relying on experience and knowledge of the patient, to determine dosages and the best treatment for each individual patient. Neither the Publisher nor the authors assume any liability for any injury and/or damage to persons or property arising from this publication.

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paper manufactured from sustainable forests

Given the complexity and quantity of clinical knowledge required to correctly identify and treat ocular disease, a quick reference text with high quality color images represents an invaluable resource to the busy clinician. Despite the availability of extensive resources online to clinicians, accessing these resources can be time consuming and often requires filtering through unnecessary information. In the exam room, facing a patient with an unfamiliar presentation or complicated medical problem, this series will be an invaluable resource.

This handy pocket sized reference series puts the knowledge of world-renowned experts at your fingertips. The standardized format provides the key element of each disease entity as your first encounter. The additional information on the clinical presentation, ancillary testing, differential diagnosis and treatment, including the prognosis, allows the clinician to instantly diagnose and treat the most common diseases seen in a busy practice. Inclusion of classical clinical color photos provides additional assurance in securing an accurate diagnosis and initiating management.

Regardless of the area of the world in which the clinician practices, these handy references guides will provide the necessary resources to both diagnose and treat a wide variety of ophthalmic diseases in all ophthalmologic specialties. The clinician who does not have easy access to sub-specialists in Anterior Segment, Glaucoma, Pediatric Ophthalmology, Strabismus, Neuro-ophthalmology, Retina, Oculoplastic and Reconstructive Surgery, and Uveitis will find these texts provide an excellent substitute. World-wide recognized experts equip the clinician with the elements needed to accurately diagnose treat and manage these complicated diseases, with confidence aided by the excellent color photos and knowledge of the prognosis.

The field of knowledge continues to expand for both the clinician in training and in practice. As a result we find it a challenge to stay up to date in the diagnosis and management of every disease entity that we face in a busy clinical practice. This series is written by an international group of experts who provide a clear, structured format with excellent photos.

It is our hope that with the aid of these six volumes, the clinician will be better equipped to diagnose and treat the diseases that affect their patients, and improve their lives.

Marian S. Macsai and Jay S. Duker

mology

Preface Series

Diagnosis Rapid

ix

If your patient has a visual problem or funny eye movements and you cannot quite figure out what is going on, chances are the problem falls within the domain of neuro-ophthalmology.

This is neuro-ophthalmology in a shot glass - short but potent. It is all here in concentrated form - the manifestations, the disease mechanisms, the pitfalls, the practical guidelines, and the pictures.

The text is bulleted for easy grasp. The fundus photographs are my best-in-show from material extending back over 40 years of clinical practice. The brain imaging illustrations are prepared to highlight the lesions. Eye movement and alignment abnormalities are a challenge to illustrate without videos; I have chosen to present schematic illustrations because still photographs so rarely tell the story properly.

I hope that you will find this book useful and that it will make you wonder at the marvels of the nervous system.

Jonathan D. Trobe

Preface

xi

Section 1

 

 

Transient Visual Loss

 

 

Transient Monocular Visual Loss (Amaurosis Fugax)

2

 

Transient Binocular Visual Loss

4

 

Migraine with Typical Visual Aura

6

 

Retinal Migraine (Presumed Retinal Vasospasm)

8

 

 

 

 

• 1 SECTIONLoss Visual Transient

Transient Monocular Visual Loss (Amaurosis

Fugax*)

Key Facts

Abrupt visual loss affecting one eye that lasts <60 min

Sometimes associated with scintillations (photopsias, positive visual phenomena)

Caused by reduced perfusion of eye (ocular transient ischemic attack, TIA)

Common causes:

cervical carotid stenosis systemic hypotension idiopathic (possible retinal artery vasospasm) impending retinal or optic nerve infarction papilledema

Evidence that carotid endarterectomy benefits patients suffering only ocular TIA is weak

Clinical Findings

Eye examination is usually normal but may show intra-arterial retinal platelet– fibrin–cholesterol (Hollenhorst) plaque, optic disc edema, or venous stasis retinopathy

Ancillary Testing

Carotid ultrasound, CT angiography, or magnetic resonance angiography to rule out stenosis, dissection, and dysplasia

Blood pressure (including orthostatic) testing to rule out hypertension or hypotension

Electrocardiography to rule out atrial fibrillation

Cardiac echography to rule out cardioembolic source

Blood tests to rule out hypercoagulable state:

complete blood count serum protein electrophoresis prothrombin and partial thromboplastin times antiphospholipid antibodies antithrombin-3

factor V Leiden prothrombin gene mutation homocysteine sickle hemoglobin serum viscosity

Differential Diagnosis

Embolism from cervical carotid artery, aortic arch, or cardiac valve or wall

Systemic hypertension or hypotension

Hypercoagulable state

Impending retinal vascular occlusion

Ischemic oculopathy

Retinal vasospasm (see Retinal migraine)

Papilledema

Treatment

Direct at underlying condition

Endarterectomy often advocated for >70% ipsilateral cervical carotid stenosis, but evidence of benefit for purely ocular TIA is weak

Reduce modifiable arteriosclerotic risk factors (diabetes, hypertension, dyslipidemia, lack of exercise, obesity, cigarette smoking)

Correct very high blood pressure but avoid excessive blood pressure lowering (may lead to perfusion failure and stroke of eyes or brain)

Aspirin 81 mg/day for underlying arteriosclerosis

Prognosis

Depends on underlying condition

*Amaurosis fugax is an old term that is out of favor because it does not specify whether transient visual loss is monocular or binocular

2

Fig. 1.1 Hollenhorst plaque. The refractile yellow dot (arrow) is an impacted platelet–fibrin embolus that traveled from the ipsilateral common carotid artery bifurcation in the neck. It produced transient visual loss by causing ischemia to the retina. Caution: most patients with transient monocular visual loss have no abnormalities on fundus examination.

Fig. 1.2 Cervical carotid stenosis. The critical narrowing of the proximal internal carotid artery (arrow) is the result of arteriosclerosis. This lesion probably gave rise to the Hollenhorst plaque in Fig. 1.1.

Fugax) (Amaurosis Loss Visual Monocular Transient

3